Background: The red ear syndrome represents a rare symptom complex consisting of auricular erythema associated with painful and burning sensations. It has been described in combination with tinnitus rarely. It has been hypothesized to be etiologically related to altered trigeminal afferent input, temporomandibular disorders, and thalamic dysfunction. Objectives: The initial objective of applying transcranial random noise stimulation (tRNS) in a case of red ear syndrome in combination with tinnitus was the alleviation of the phantom sounds. Study Design: This is a case report on the successful treatment of red ear syndrome with tinnitus by means of transcranial random noise stimulation (tRNS) and a short review on the published cases of this condition. Setting: We present the case of a 50-year-old woman reporting a simultaneous onset of constant left-sided tinnitus and feelings of warmth accompanied by an intermittent stabbing and/or oppressive pain stretching from the ipsilateral ear to the head/neck/shoulder region, occasionally accompanied by nausea/vomiting and dizziness. After failure of pharmacological treatment attempts, either because of lacking clinical effects (gabapentin, zolmitriptan, and indomethacin) or because of adverse reactions (pregabaline), the patient was offered an experimental neuromodulatory treatment with bitemporal tRNS primarily targeting the tinnitus complaints of the patient. Methods: tRNS was conducted in 2 – 3 day sessions (stimulation site: bilateral temporal cortex/2.0 mA/10 s on-and-off-ramp/offset 0 mA/20 min/random frequencies 101 – 640Hz / NeuroConn Eldith DC-Stimulator plus). Results: In 3 consecutive pain attacks repeated sessions of tRNS resulted in substantial alleviation of pain intensity and a prolongation of the interval between attacks. This was an expected finding as the proposed tRNS treatment was initially offered to the patient aiming at an alleviation of the tinnitus complaints (which remained unaffected by tRNS). Limitations: The reported data derive from compassionate use treatment in one single patient. Application of a sham condition would have been desirable, but is not possible in the context of compassionate use treatment. Nevertheless, we would consider it rather unlikely that the reported effects are purely unspecific as the patient did exclusively report symptom alleviation of pain-related parameters without affecting the tinnitus. Conclusions: This case report demonstrates the feasibility and therapeutic potential of applying neuromodulatory treatment approaches in red ear syndrome, a rare form of trigemino-autonomal headache. Therefore, it deserves detailed observation in clinical routine applications as well as controlled trials further investigating its neurobiological effects. Key words: Red ear syndrome, pain, trigemino-autonomal headache, chronic tinnitus, transcranial electrical stimulation, random noise stimulation